Behavioral variant of frontotemporal dementia mimicking Huntington's disease

T Rune Nielsen; Peter Bruhn; Jørgen E Nielsen; Lena E Hjermind

doi:10.1017/s1041610210000098

Behavioral variant of frontotemporal dementia mimicking Huntington's disease

T Rune Nielsen, Peter Bruhn, Jørgen E Nielsen, Lena E Hjermind

Medical Genetics Program

9 Citations (Scopus)

Abstract

Behavioral changes and cognitive decline are the core clinical manifestations in the behavioral variant of frontotemporal dementia (bv-FTD). The behavioral changes may include characteristic stereotypic movements. These movements, although without clear purpose, are not involuntary. Involuntary movements are usually not seen in FTD. Two patients with involuntary choreoathetoid movements but otherwise presenting a bv-FTD-phenotype were referred and Huntington's disease (HD) was suspected. The diagnoses of bv-FTD were made after comprehensive assessment and exclusion of other diagnoses, including HD and Huntington's disease-like (HDL) phenotypes. Although a definite diagnosis will require neuropathological confirmation, we conclude that a HDL phenotype may be part of the clinical spectrum of the bv-FTD phenotype.

Original language	English
Journal	International Psychogeriatrics
Volume	22
Issue number	4
Pages (from-to)	674-7
Number of pages	3
ISSN	1041-6102
DOIs	https://doi.org/10.1017/s1041610210000098
Publication status	Published - Jun 2010

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title = "Behavioral variant of frontotemporal dementia mimicking Huntington's disease",

abstract = "Behavioral changes and cognitive decline are the core clinical manifestations in the behavioral variant of frontotemporal dementia (bv-FTD). The behavioral changes may include characteristic stereotypic movements. These movements, although without clear purpose, are not involuntary. Involuntary movements are usually not seen in FTD. Two patients with involuntary choreoathetoid movements but otherwise presenting a bv-FTD-phenotype were referred and Huntington's disease (HD) was suspected. The diagnoses of bv-FTD were made after comprehensive assessment and exclusion of other diagnoses, including HD and Huntington's disease-like (HDL) phenotypes. Although a definite diagnosis will require neuropathological confirmation, we conclude that a HDL phenotype may be part of the clinical spectrum of the bv-FTD phenotype.",

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AU - Nielsen, T Rune

AU - Bruhn, Peter

AU - Nielsen, Jørgen E

AU - Hjermind, Lena E

PY - 2010/6

Y1 - 2010/6

N2 - Behavioral changes and cognitive decline are the core clinical manifestations in the behavioral variant of frontotemporal dementia (bv-FTD). The behavioral changes may include characteristic stereotypic movements. These movements, although without clear purpose, are not involuntary. Involuntary movements are usually not seen in FTD. Two patients with involuntary choreoathetoid movements but otherwise presenting a bv-FTD-phenotype were referred and Huntington's disease (HD) was suspected. The diagnoses of bv-FTD were made after comprehensive assessment and exclusion of other diagnoses, including HD and Huntington's disease-like (HDL) phenotypes. Although a definite diagnosis will require neuropathological confirmation, we conclude that a HDL phenotype may be part of the clinical spectrum of the bv-FTD phenotype.

AB - Behavioral changes and cognitive decline are the core clinical manifestations in the behavioral variant of frontotemporal dementia (bv-FTD). The behavioral changes may include characteristic stereotypic movements. These movements, although without clear purpose, are not involuntary. Involuntary movements are usually not seen in FTD. Two patients with involuntary choreoathetoid movements but otherwise presenting a bv-FTD-phenotype were referred and Huntington's disease (HD) was suspected. The diagnoses of bv-FTD were made after comprehensive assessment and exclusion of other diagnoses, including HD and Huntington's disease-like (HDL) phenotypes. Although a definite diagnosis will require neuropathological confirmation, we conclude that a HDL phenotype may be part of the clinical spectrum of the bv-FTD phenotype.

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DO - 10.1017/s1041610210000098

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JO - International Psychogeriatrics

JF - International Psychogeriatrics

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Behavioral variant of frontotemporal dementia mimicking Huntington's disease

Abstract

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